Mid-aortic syndrome is a rare condition. It is characterized by narrowing of distal descending thoracic or abdominal aorta, usually with involvement of renal arteries and other visceral branches of aorta.

Material and methods:

We analyzed the onset, diagnostic evaluation, treatment and outcome in three children with mid-aortic syndrome, operated at the University Children’s Hospital, Ljubljana, Slovenia.

Results:

Three children, aged 3,5, 5, and 12 years were referred to our hospital due to grade 2. hypertension in two boys and hypertensive urgency in a girl. In all of them a bruit on the renal arteries could be heard and plasma renin activity was elevated. Concentric hypertrophy was shown in all of them, with left ventricular dysfunction in two. By Renal Doppler Ultrasound aortic and renal artery stenosis were suspected, while MR angiography and digital subtraction angiography confirmed the diagnosis of mid-aortic syndrome. The surgery was performed in all three children due to inefficient medical and endovascular treatment in controlling hypertension. Aorto-aortic bypass grafting, resection of the stenotic segments of both renal arteries and implantation of the arterial segments into the graft was performed in first case. In second case surgical reconstruction of abdominal aorta and renal arteries with core matrix patch was performed and stents in both right renal arteries were inserted. In the last patient reconstruction of abdominal aorta with bio-patch and aorto-renal bypass with auto-venous graft was performed.

2, 3, and 7 years after surgery hypertension is well under control and left ventricular hypertrophy and dysfunction subsided in all three patients, receiving 2 to 4 antihypertensive drugs. All have normal renal function, development and growth.

Conclusions:

Surgical treatment of mid-aortic syndrome is an option in children in whom medical and endovascular treatment alone cannot sufficiently control blood pressure. It could however be technically challenging, especially in small children.